Metreleptin Treatment in Three Patients with Generalized Lipodystrophy.

Generalized lipodystrophy (GL) is a rare inherited or acquired disease characterized by widespread loss of subcutaneous fat, leading to leptin deficiency, ectopic fat deposition, and severe metabolic abnormalities. Previous studies have shown the benefit of leptin replacement (metreleptin) in ameliorating metabolic complications, but little is known about the experience of metreleptin treatment outside of a research setting. We report on post-marketing clinical experience with metreleptin therapy in three patients with GL and marked hypoleptinemia, uncontrolled diabetes, and hypertriglyceridemia.

Relationship between cumulative BMI and symptomatic, psychosocial, and medical outcomes in patients with borderline personality disorder.

We examined the relationship between cumulative body mass index (BMI) and symptomatic, psychosocial, and medical outcomes in patients with borderline personality disorder (BPD). Two hundred female borderline patients were weighed and measured during their index admission. They were subsequently interviewed at six-, eight-, and 10-year intervals.

[The calcium sensitizer levosimendan gives superior results to dobutamine in postoperative low cardiac output syndrome].

Introduction And Objectives: The use of levosimendan to treat postoperative low cardiac output syndrome (LCOS) has been studied in only small patient series and in randomized trials focusing on hemodynamic variables. The objective of the present study was to assess the effectiveness of levosimendan, compared with dobutamine, as a treatment for postoperative LCOS.

Methods: Patients with LCOS were randomly assigned to receive either levosimendan (loading dose, 10 microg/kg, followed by 0.

Metformin reverts deleterious effects of advanced glycation end-products (AGEs) on osteoblastic cells.

Advanced glycation endproducts (AGEs) are implicated in the complications of diabetes and ageing, affecting several tissues, including bone. Metformin, an insulin-sensitizer drug, reduces the risk of life-threatening macrovascular complications. We have evaluated the hypothesis that metformin can abrogate AGE-induced deleterious effects in osteoblastic cells in culture.

Lipoprotein alterations, hepatic lipase activity, and insulin sensitivity in subclinical hypothyroidism: response to L-T(4) treatment.

Unlabelled: Subclinical hypothyroidism (sH) has been associated with atherosclerotic cardiovascular disease even in the absence of hypercholesterolemia.

Objective: Our study was designed to assess the hypothesis that other pro-atherogenic parameters, such as qualitative lipoprotein changes and insulin resistance, might be present in sH.

Design And Methods: Twenty-one sH women were compared to 11 female controls matched for body mass index, menopausal status, and age.

[Semantic dementia, a many-worded disorder].

Introduction: Semantic dementia is a progressive, relatively selective disorder affecting the semantic system with involvement of the verbal and non-verbal functions. The clinical picture is well characterised despite the confusion that may be generated by the different ways of classifying it. AIM.

Osteogenic actions of the anti-diabetic drug metformin on osteoblasts in culture.

An association has been previously established between uncompensated diabetes mellitus and the loss of bone mineral density and/or quality. In this study, we evaluated the effects of metformin on the growth and differentiation of osteoblasts in culture. Treatment of two osteoblast-like cells (UMR106 and MC3T3E1) with metformin (25-500 microM) for 24 h led to a dose-dependent increase of cell proliferation.

Infectious keratitis in the corneal graft: treatment with partial conjunctival flaps.

Background And Objective: The infection of the corneal graft is one of the most serious complications following keratoplasty. In many instances, it can be treated successfully with intensive topical and subconjunctival antibiotics. However, when this therapy is ineffective, a surgical approach must be considered.

Distance correction in the electrocardiographic estimation of left ventricular mass.

Assessment of the left ventricular mass (LVM) from electrocardiograms may be improved by the addition of clinical variables into a multivariate equation. As the heart-thorax distance may affect the results, its relationships with electrocardiographic and clinical data have been evaluated in a group of 220 subjects (53 +/- 15 years, 126 female, 175 without demonstrated heart disease) who were assessed for echocardiographic LVM and heart-thorax distance. Sokolow, Cornell, and total QRS voltage indexes were obtained.

[The pharmacology of vestibular disorders].

Introduction: In recent years, significant advances have been made in the physiology and the pharmacology of vestibular disorders. It is now possible to elaborate an approximation of the mechanisms of action of the drugs used in the symptomatic treatment of vertigo.

Development: These drugs usually have a number of different pharmacological actions and overlapping effects.

[A survey of sleeping difficulties in an urban Latin American population].

Aims: To report the results of the first regional survey on the prevalence of sleep disturbances in an urban Latin American population.

Subjects And Methods: Door-to-door and telephone interviews in main urban areas were performed in two phases. In phase one, 1776 adults, aged 18 to 70 were interviewed in Buenos Aires and São Paulo.

[Neck vibration: usefulness in neurotology].

Introduction: Recently, mechanical vibration on the neck has begun to be used to improve diagnostic sensitivity in patients who have vestibular disorders.

Method: We review results of studies of this stimulus in normal subjects and patients with vestibular lesions: the ocular responses, changes in the subjective straight ahead, and postural changes generated by the neck vibration.

Conclusion: Although the underlying mechanism for the responses to neck vibration remains uncertain, it is an excellent addition to the neurotologic exam since it allows us demonstrate vestibular deficit with greater sensitivity than other maneuvers.

Attitudes towards treatment among patients suffering from sleep disorders. A Latin American survey.

Background: Although sleep disorders are common, they frequently remain unnoticed by the general practitioner. Few data are available about the willingness and reasons of patients with sleep disturbances to seek for medical assistance.

Methods: The results of a cross-sectional community-based multinational survey in three major Latin American urban areas, i.

Efficacy and safety of levodopa with entacapone in Parkinson's disease patients suboptimally controlled with levodopa alone, in daily clinical practice: an international, multicentre, open-label study.

The combination of entacapone with levodopa is effective in the treatment of Parkinson's disease (PD), providing significant improvements in 'on' time and Unified Parkinson's Disease Rating Scale (UPDRS) motor and ADL scores in controlled clinical trials. This multicentre, open-label study was designed to further evaluate the effectiveness of levodopa combined with entacapone 200 mg in routine clinical practice. Patients experiencing end-of-dose wearing-off were treated for 8 weeks (treatment phase), with an optional extension phase up to 20 weeks.

Early onset parkinsonism.

Early-onset parkinsonism refers to those patients with onset of a parkinsonian syndrome before age 40. These cases can be further subdivided into Young-onset (between ages 40 and 21) and Juvenile-onset (below age 21). In this review we will focus on the peculiar clinical features that distinguish these cases from classic late-onset parkinsonism, their characteristic response to medication, and their heterogeneous pathogenesis, including recent findings in the field of genetics that would suggest that a significant number of these patients are the result of genetic mutations.

[Psychiatric aspects of Epilepsy].

The classification of epileptic seizures is reviewed, emphasizing the clinical features of partial seizures with psychic symptoms. Psychiatric issues in epilepsy are also assessed, particularly affective disorders and psychosis. Although there currently is no internationally accepted syndromic classification for them, we expose recently proposed definitions.

Evaluation of innovative skin-marking technique performed before thyroid ultrasound-guided fine-needle aspiration biopsies.

Objective: To introduce an innovative skin-marking technique performed before ultrasound-guided fine-needle aspiration biopsy (US-FNAB) of the thyroid.

Methods: We studied 248 patients with thyroid nodules, who were classified on the basis of physical examination into two groups-those with palpable nodules (N = 127) and those with nonpalpable or difficult-to-palpate nodules (N = 121). Each group was further subdivided according to the size of the thyroid nodule (< or = 15 mm versus >15 mm).

Characterization of sex hormone-binding globulin isoforms in hypothyroid women.

Liver sex hormone-binding globulin (SHBG) biosynthesis is regulated by triiodothyronine (T3). This regulation is responsible for increased serum SHBG concentrations in hyperthyroid states. However, in hypothyroidism, normal SHBG levels are frequently found.

[Psychiatric comorbidity in clinical Tourette Syndrome's samples].

Classically conceived essentially as a rare neurological disorder, Tourette Syndrome (TS) is frequently associated with comorbid disorders such as attention deficit, obsessive-compulsive disorder, impulse control problem and a variety of other behavioral symptoms that can clearly have an impact on cognitive,educational and psychosocial function and can be more debilitating than the cardinal motor features. The full spectrum of TS is often not recognized in our medical community while it's usual atributtion to exclusive pshycogenic causes leads to underdiagnosis. Meanwhile epidemiological studies shows it's increasingly prevalence and its pottencially multiple deficits, specially on pediatrical clinical population.

Estrogenic status influences nitric oxide-regulated TNF-alpha release from human peripheral blood monocytes.

Cytokines and nitric oxide (NO) have been implicated in bone loss caused by estrogen deficiency. Here we evaluated the effect of nitric oxide synthase (NOS) inhibitors on the bone particle resorbing activity and TNF-alpha release of cultured peripheral blood monocytes (PBM) obtained from 10 premenopausal (PreM) and 10 postmenopausal (PostM) women. Gonadal status (menopause < 3 yr) was assessed by FSH and estradiol.

Shortening the time to the diagnosis of amyotrophic lateral sclerosis: role of the neurologist.

Early diagnosis is particularly relevant in the diagnosis of amyotrophic lateral sclerosis (ALS) as new therapies, which may have a direct impact on the outcome of the disease, are being developed. Many ALS patients go to non-neurological specialists rather than neurologists for their first consultation. Neurologists specialized in the field of neuromuscular diseases therefore have a duty to help general neurologists and other non-neurological specialists to recognize the disease.

Penetrating keratoplasty in eyes treated with conjunctival flaps.

Purpose: To analyze the results of corneal transplants in severely inflamed eyes previously treated with conjunctival flaps.

Methods: In the period 1984-1996, seven homoplastic penetrating keratoplasties (PKs) and one rotating autokeratoplasty were performed on a series of 47 eyes of 46 patients treated with partial or total conjunctival flaps. Two of these PKs were combined with an extracapsular cataract extraction, and the rotating autograft was combined with an extracapsular cataract extraction and intraocular lens implantation.

[Diagnosis and treatment of coagulation disorders].

Introduction: Approximately 5% of all cerebrovascular events (CVE), and 10% of those occurring in young patients, are due to hematological disorders. Hypercoagulability states are related to CVE in young patients, deep vein thrombosis, recurrent thromboses, pulmonary embolism, a family history of thrombosis and unusual venous and arterial thromboses.

Development: The conditions related to increased risk of thrombosis are: the congenital thrombophilias due to deficiency of protein C, protein S or antithrombin III, resistance to protein C activated by Leiden's factor V--cofactor of protein C with genetic mutation--; the primary antiphospholipid syndrome with anticardiolipin antibodies and lupus inhibitor; platelet disorders, deficit of heparin cofactor II, deficit of plasminogen and plasminogen tissue activator (t-PA) and increase in the inhibitor of plasminogen tissue activator (PAI-I); alterations in factors of coagulation such as deficits of factor VII and factor XIII, mutation of prothrombin 20210-->A, increase in factor VIII.